TY  - JOUR
A1  - Wohnrade, Camilla
A1  - Velling, Ann-Kathrin
A1  - Mix, Lucas
A1  - Wurster, Claudia D.
A1  - Cordts, Isabell
A1  - Stolte, Benjamin
A1  - Zeller, Daniel
A1  - Uzelac, Zeljko
A1  - Platen, Sophia
A1  - Hagenacker, Tim
A1  - Deschauer, Marcus
A1  - Lingor, Paul
A1  - Ludolph, Albert C.
A1  - Lulé, Dorothée
A1  - Petri, Susanne
A1  - Osmanovic, Alma
A1  - Schreiber-Katz, Olivia
T1  - Health-related quality of life in spinal muscular atrophy patients and their caregivers — a prospective, cross-sectional, multi-center analysis
JF  - Brain Sciences
N2  - Spinal muscular atrophy (SMA) is a disabling disease that affects not only the patient’s health-related quality of life (HRQoL), but also causes a high caregiver burden (CGB). The aim of this study was to evaluate HRQoL, CGB, and their predictors in SMA. In two prospective, cross-sectional, and multi-center studies, SMA patients (n = 39) and SMA patient/caregiver couples (n = 49) filled in the EuroQoL Five Dimension Five Level Scale (EQ-5D-5L) and the Short Form Health Survey 36 (SF-36). Caregivers (CGs) additionally answered the Zarit Burden Interview (ZBI) and the Hospital Anxiety and Depression Scale (HADS). Patients were clustered into two groups with either low or high HRQoL (EQ-5D-5L index value <0.259 or >0.679). The latter group was mostly composed of ambulatory type III patients with higher motor/functional scores. More severely affected patients reported low physical functioning but good mental health and vitality. The CGB (mean ZBI = 22/88) correlated negatively with patients’ motor/functional scores and age. Higher CGB was associated with a lower HRQoL, higher depression and anxiety, and more health impairments of the CGs. We conclude that patient and CG well-being levels interact closely, which highlights the need to consider the health of both parties while evaluating novel treatments.
KW  - caregiver
KW  - caregiver burden
KW  - mental health
KW  - quality of life
KW  - spinal muscular atrophy
KW  - patient reported outcome measures
Y1  - 2023
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-305048
SN  - 2076-3425
VL  - 13
IS  - 1
ER  - 
TY  - JOUR
A1  - Lulé, Dorothée
A1  - Kübler, Andrea
A1  - Ludolph, Albert C.
T1  - Ethical principles in patient-centered medical care to support quality of life in amyotrophic lateral sclerosis
JF  - Frontiers in Neurology
N2  - It is one of the primary goals of medical care to secure good quality of life (QoL) while prolonging survival. This is a major challenge in severe medical conditions with a prognosis such as amyotrophic lateral sclerosis (ALS). Further, the definition of QoL and the question whether survival in this severe condition is compatible with a good QoL is a matter of subjective and culture-specific debate. Some people without neurodegenerative conditions believe that physical decline is incompatible with satisfactory QoL. Current data provide extensive evidence that psychosocial adaptation in ALS is possible, indicated by a satisfactory QoL. Thus, there is no fatalistic link of loss of QoL when physical health declines. There are intrinsic and extrinsic factors that have been shown to successfully facilitate and secure QoL in ALS which will be reviewed in the following article following the four ethical principles (1) Beneficence, (2) Non-maleficence, (3) Autonomy and (4) Justice, which are regarded as key elements of patient centered medical care according to Beauchamp and Childress. This is a JPND-funded work to summarize findings of the project NEEDSinALS (www.NEEDSinALS.com) which highlights subjective perspectives and preferences in medical decision making in ALS.
KW  - ethics
KW  - quality of life (QoL)
KW  - care
KW  - amyotrophic lateral sclerosis (ALS)
KW  - well-being
KW  - depression
KW  - coping
KW  - psychosocial adaptation
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-196239
SN  - 1664-2295
VL  - 10
ER  -